Akinetic mutism

Last updated
Akinetic mutism
Specialty Neurology, Psychiatry

Akinetic mutism is a medical condition where patients tend neither to move (akinesia) nor speak (mutism). It is the most extreme disorder of diminished motivation. Akinetic mutism was first described in 1941 as a mental state where patients lack the ability to move or speak. [1] However, their eyes may follow their observer or be diverted by sound. [1] Patients lack most motor functions such as speech, facial expressions, and gestures, but demonstrate apparent alertness. [2] They exhibit reduced activity and slowness, and can speak in whispered monosyllables. [1] [3] Patients often show visual fixation on their examiner, move their eyes in response to an auditory stimulus, or move after often repeated commands. [1] [2] Patients with akinetic mutism are not paralyzed, but lack the will to move. [1] Many patients describe that as soon as they "will" or attempt a movement, a "counter-will" or "resistance" rises up to meet them. [4]

Contents

Types

Frontal akinetic mutism can occur after a frontal lobe injury Gehirn, lateral - Lobi eng.svg
Frontal akinetic mutism can occur after a frontal lobe injury
The mesencephalic form of akinetic mutism occurs in the midbrain (4) Human brain midsagittal cut description.JPG
The mesencephalic form of akinetic mutism occurs in the midbrain (4)

Akinetic mutism varies across all patients. Its form, intensity, and clinical features correspond more closely to its functional anatomy rather than to its pathology. However, akinetic mutism most often appears in two different forms: frontal and mesencephalic. [2]

Frontal akinetic mutism

Akinetic mutism can occur in the frontal region of the brain and occurs because of bilateral frontal lobe damage. Akinetic mutism as a result of frontal lobe damage is clinically characterized as hyperpathic. [5] It occurs in patients with bilateral circulatory disturbances in the supply area of the anterior cerebral artery. [2]

Mesencephalic akinetic mutism

Akinetic mutism can also occur as a result of damage to the mesencephalic region of the brain. Mesencephalic akinetic mutism is clinically categorized as somnolent or apathetic akinetic mutism. [5] It is characterized by vertical gaze palsy and ophthalmoplegia. This state of akinetic mutism varies in intensity, but it is distinguished by drowsiness, lack of motivation, hyper-somnolence, and reduction in spontaneous verbal and motor actions. [2] [5]

Symptoms and signs

Causes

Many cases of akinetic mutism have occurred after a thalamic stroke. Thalamus.png
Many cases of akinetic mutism have occurred after a thalamic stroke.

Akinetic mutism can be caused by a variety of things. It often occurs after brain injury or as a symptom of other diseases.

Frontal lobe damage

Akinetic mutism is often the result of severe frontal lobe injury in which the pattern of inhibitory control is one of increasing passivity and gradually decreasing speech and motion.[ citation needed ]

Thalamic stroke

Many cases of akinetic mutism occur after a thalamic stroke. [3] The thalamus helps regulate consciousness and alertness.[ citation needed ]

Ablation of cingulate gyrus

Another cause of both akinesia and mutism is ablation of the cingulate gyrus. Destruction of the cingulate gyrus has been used in the treatment of psychosis. Such lesions result in akinesia, mutism, apathy, and indifference to painful stimuli. [7] The anterior cingulate cortex is thought to supply a "global energizing factor" that stimulates decision making. [8] When the anterior cingulate cortex is damaged, it can result in akinetic mutism.[ citation needed ]

Other

Akinetic mutism is a symptom during the final stages of Creutzfeldt–Jakob disease (a rare degenerative brain disease) and can help diagnose patients with this disease. [2] [9] It can also occur in a stroke that affects both anterior cerebral artery territories.[ citation needed ] Another cause is neurotoxicity due to exposure to certain drugs such as tacrolimus and cyclosporine.

Other causes of akinetic mutism are as follows:

Diagnosis

Akinetic mutism can be misdiagnosed as depression, delirium, or locked-in syndrome, all of which are common following a stroke. [3] Patients with depression can experience apathy, slurring of speech, and body movements similar to akinetic mutism. Similarly to akinetic mutism, patients with locked-in syndrome experience paralysis and can only communicate with their eyes. [3] Correct diagnosis is important to ensure proper treatment. A variety of treatments for akinetic mutism have been documented, but treatments vary between patients and cases.[ citation needed ]

Treatment

Magnesium sulfate

Treatments using intravenous magnesium sulfate have shown to reduce the symptoms of akinetic mutism. In one case, a 59-year-old woman was administered intravenous magnesium sulfate in an attempt to resolve her akinetic mutism. The patient was given 500 mg of magnesium every eight hours, and improvement was seen after 24 hours. She became more verbal and attentive, and treatment was increased to 1000 mg every eight hours as conditions continued to improve. [11]

Cyst puncture

As seen in the case of Elsie Nicks, the puncture or removal of a cyst causing akinetic mutism can relieve symptoms almost immediately. However, if the cyst fills up again, the symptoms can reappear. [1]

Dopamine agonist therapy

Symptoms of akinetic mutism suggest a possible presynaptic deficit in the nigrostriatal pathway, which transmits dopamine. Some patients with akinetic mutism have shown to improve with levodopa or dopamine agonist therapy, [12] or by repleting dopamine in the motivational circuit with stimulants, antidepressants, or agonists such as bromocriptine or amantadine. [6]

Other treatments include amantadine, carbidopa-levodopa, donepezil, memantine, and oral magnesium oxide. [6] [11]

History

Fourteen-year-old Elsie Nicks was the first patient to be diagnosed with akinetic mutism by Hugh Cairns in 1941. She suffered from severe headaches her entire life and was eventually given morphine to help with treatment. She began to enter a state of akinetic mutism, experiencing apathy and loss of speech and motor control. A cyst on her right lateral ventricle was tapped, and as soon as the needle advanced toward the cyst, she let out a loud noise and was able to state her name, age, and address. After her cyst was emptied, she regained her alertness and intelligence, and she had no recollection of her time spent in the hospital. The cyst was drained two more times over the next seven months and was eventually removed. After eight months of rehabilitation, Elsie no longer experienced headaches or akinetic mutism symptoms. [1]

See also

Related Research Articles

<span class="mw-page-title-main">Catatonia</span> Psychiatric behavioural syndrome

Catatonia is a complex neuropsychiatric behavioral syndrome that is characterized by abnormal movements, immobility, abnormal behaviors, and withdrawal. The onset of catatonia can be acute or subtle and symptoms can wax, wane, or change during episodes. It has historically been related to schizophrenia, but catatonia is most often seen in mood disorders. It is now known that catatonic symptoms are nonspecific and may be observed in other mental, neurological, and medical conditions. Catatonia is now a stand-alone diagnosis, and the term is used to describe a feature of the underlying disorder.

Bilateral cingulotomy is a form of psychosurgery, introduced in 1948 as an alternative to lobotomy. Today, it is mainly used in the treatment of depression and obsessive-compulsive disorder. In the early years of the twenty-first century, it was used in Russia to treat addiction. It is also used in the treatment of chronic pain. The objective of this procedure is the severing of the supracallosal fibres of the cingulum bundle, which pass through the anterior cingulate gyrus.

<span class="mw-page-title-main">Hydrocephalus</span> Abnormal increase in cerebrospinal fluid in the ventricles of the brain

Hydrocephalus is a condition in which an accumulation of cerebrospinal fluid (CSF) occurs within the brain. This typically causes increased pressure inside the skull. Older people may have headaches, double vision, poor balance, urinary incontinence, personality changes, or mental impairment. In babies, it may be seen as a rapid increase in head size. Other symptoms may include vomiting, sleepiness, seizures, and downward pointing of the eyes.

Porencephaly is an extremely rare cephalic disorder involving encephalomalacia. It is a neurological disorder of the central nervous system characterized by cysts or cavities within the cerebral hemisphere. Porencephaly was termed by Heschl in 1859 to describe a cavity in the human brain. Derived from Greek roots, the word porencephaly means 'holes in the brain'. The cysts and cavities are more likely to be the result of destructive (encephaloclastic) cause, but can also be from abnormal development (malformative), direct damage, inflammation, or hemorrhage. The cysts and cavities cause a wide range of physiological, physical, and neurological symptoms. Depending on the patient, this disorder may cause only minor neurological problems, without any disruption of intelligence, while others may be severely disabled or die before the second decade of their lives. However, this disorder is far more common within infants, and porencephaly can occur both before or after birth.

<span class="mw-page-title-main">Apathy</span> State of indifference, or the suppression of emotions

Apathy, also referred to as indifference, is a lack of feeling, emotion, interest, or concern about something. It is a state of indifference, or the suppression of emotions such as concern, excitement, motivation, or passion. An apathetic individual has an absence of interest in or concern about emotional, social, spiritual, philosophical, virtual, or physical life and the world. Apathy can also be defined as a person's lack of goal orientation. Apathy falls in the less extreme spectrum of diminished motivation, with abulia in the middle and akinetic mutism being more extreme than both apathy and abulia.

<span class="mw-page-title-main">Caudate nucleus</span> Structure of the striatum in the basal ganglia of the brain

The caudate nucleus is one of the structures that make up the corpus striatum, which is a component of the basal ganglia in the human brain. While the caudate nucleus has long been associated with motor processes due to its role in Parkinson's disease, it plays important roles in various other nonmotor functions as well, including procedural learning, associative learning and inhibitory control of action, among other functions. The caudate is also one of the brain structures which compose the reward system and functions as part of the cortico-basal ganglia-thalamo-cortical loop.

<span class="mw-page-title-main">Frontal lobe</span> Part of the brain

The frontal lobe is the largest of the four major lobes of the brain in mammals, and is located at the front of each cerebral hemisphere. It is parted from the parietal lobe by a groove between tissues called the central sulcus and from the temporal lobe by a deeper groove called the lateral sulcus. The most anterior rounded part of the frontal lobe is known as the frontal pole, one of the three poles of the cerebrum.

In neurology, abulia, or aboulia, refers to a lack of will or initiative and can be seen as a disorder of diminished motivation. Abulia falls in the middle of the spectrum of diminished motivation, with apathy being less extreme and akinetic mutism being more extreme than abulia. The condition was originally considered to be a disorder of the will, and aboulic individuals are unable to act or make decisions independently; and their condition may range in severity from subtle to overwhelming. In the case of akinetic mutism, many patients describe that as soon as they "will" or attempt a movement, a "counter-will" or "resistance" rises up to meet them.

<span class="mw-page-title-main">Progressive supranuclear palsy</span> Medical condition

Progressive supranuclear palsy (PSP) is a late-onset neurodegenerative disease involving the gradual deterioration and death of specific volumes of the brain. The condition leads to symptoms including loss of balance, slowing of movement, difficulty moving the eyes, and cognitive impairment. PSP may be mistaken for other types of neurodegeneration such as Parkinson's disease, frontotemporal dementia and Alzheimer's disease. The cause of the condition is uncertain, but involves the accumulation of tau protein within the brain. Medications such as levodopa and amantadine may be useful in some cases.

<span class="mw-page-title-main">Interventricular foramina (neuroanatomy)</span> It is part of diencephalon that makes connection between lateral and third ventricular

In the brain, the interventricular foramina are channels that connect the paired lateral ventricles with the third ventricle at the midline of the brain. As channels, they allow cerebrospinal fluid (CSF) produced in the lateral ventricles to reach the third ventricle and then the rest of the brain's ventricular system. The walls of the interventricular foramina also contain choroid plexus, a specialized CSF-producing structure, that is continuous with that of the lateral and third ventricles above and below it.

<span class="mw-page-title-main">Anterior cerebral artery</span> Artery supplying the brain

The anterior cerebral artery (ACA) is one of a pair of cerebral arteries that supplies oxygenated blood to most midline portions of the frontal lobes and superior medial parietal lobes of the brain. The two anterior cerebral arteries arise from the internal carotid artery and are part of the circle of Willis. The left and right anterior cerebral arteries are connected by the anterior communicating artery.

<span class="mw-page-title-main">Hypokinesia</span> Decreased movement due to basal ganglia dysfunction

Hypokinesia is one of the classifications of movement disorders, and refers to decreased bodily movement. Hypokinesia is characterized by a partial or complete loss of muscle movement due to a disruption in the basal ganglia. Hypokinesia is a symptom of Parkinson's disease shown as muscle rigidity and an inability to produce movement. It is also associated with mental health disorders and prolonged inactivity due to illness, amongst other diseases.

<span class="mw-page-title-main">Corticobasal degeneration</span> Rare neurodegenerative disease

Corticobasal degeneration (CBD) is a rare neurodegenerative disease involving the cerebral cortex and the basal ganglia. CBD symptoms typically begin in people from 50 to 70 years of age, and typical survival before death is eight years. It is characterized by marked disorders in movement and cognition, and is classified as one of the Parkinson plus syndromes. Diagnosis is difficult, as symptoms are often similar to those of other disorders, such as Parkinson's disease, progressive supranuclear palsy, and dementia with Lewy bodies, and a definitive diagnosis of CBD can only be made upon neuropathologic examination.

<span class="mw-page-title-main">Frontal lobe disorder</span> Brain disorder

Frontal lobe disorder, also frontal lobe syndrome, is an impairment of the frontal lobe of the brain due to disease or frontal lobe injury. The frontal lobe plays a key role in executive functions such as motivation, planning, social behaviour, and speech production. Frontal lobe syndrome can be caused by a range of conditions including head trauma, tumours, neurodegenerative diseases, neurodevelopmental disorders, neurosurgery and cerebrovascular disease. Frontal lobe impairment can be detected by recognition of typical signs and symptoms, use of simple screening tests, and specialist neurological testing.

Neuroepithelial cells, or neuroectodermal cells, form the wall of the closed neural tube in early embryonic development. The neuroepithelial cells span the thickness of the tube's wall, connecting with the pial surface and with the ventricular or lumenal surface. They are joined at the lumen of the tube by junctional complexes, where they form a pseudostratified layer of epithelium called neuroepithelium.

Athymhormic syndrome, psychic akinesia, or auto-activation deficit (AAD) is a rare psychopathological and neurological syndrome characterized by extreme passivity, apathy, blunted affect and a profound generalized loss of self-motivation and conscious thought. For example, a patient spent 45 minutes with his hands on a lawn mower, totally unable to initiate the act of mowing. This "kinetic blockade" disappeared instantaneously when his son told him to move. The existence of such symptoms in patients after damage to certain structures in the brain has been used in support of a physical model of motivation in human beings, wherein the limbic loop of the basal ganglia is the initiator of directed action and thought. It is a disorder of diminished motivation.

<span class="mw-page-title-main">Foix–Chavany–Marie syndrome</span> Medical condition

Foix–Chavany–Marie syndrome (FCMS), also known as bilateral opercular syndrome, is a neuropathological disorder characterized by paralysis of the facial, tongue, pharynx, and masticatory muscles of the mouth that aid in chewing. The disorder is primarily caused by thrombotic and embolic strokes, which cause a deficiency of oxygen in the brain. As a result, bilateral lesions may form in the junctions between the frontal lobe and temporal lobe, the parietal lobe and cortical lobe, or the subcortical region of the brain. FCMS may also arise from defects existing at birth that may be inherited or nonhereditary. Symptoms of FCMS can be present in a person of any age and it is diagnosed using automatic-voluntary dissociation assessment, psycholinguistic testing, neuropsychological testing, and brain scanning. Treatment for FCMS depends on the onset, as well as on the severity of symptoms, and it involves a multidisciplinary approach.

<span class="mw-page-title-main">Cerebral shunt</span> Surgical implant to treat hydrocephalus

A cerebral shunt is a device permanently implanted inside the head and body to drain excess fluid away from the brain. They are commonly used to treat hydrocephalus, the swelling of the brain due to excess buildup of cerebrospinal fluid (CSF). If left unchecked, the excess CSF can lead to an increase in intracranial pressure (ICP), which can cause intracranial hematoma, cerebral edema, crushed brain tissue or herniation. The drainage provided by a shunt can alleviate or prevent these problems in patients with hydrocephalus or related diseases.

Cerebellar cognitive affective syndrome (CCAS), also called Schmahmann's syndrome is a condition that follows from lesions (damage) to the cerebellum of the brain. It refers to a constellation of deficits in the cognitive domains of executive function, spatial cognition, language, and affect resulting from damage to the cerebellum. Impairments of executive function include problems with planning, set-shifting, abstract reasoning, verbal fluency, and working memory, and there is often perseveration, distractibility and inattention. Language problems include dysprosodia, agrammatism and mild anomia. Deficits in spatial cognition produce visual–spatial disorganization and impaired visual–spatial memory. Personality changes manifest as blunting of affect or disinhibited and inappropriate behavior. These cognitive impairments result in an overall lowering of intellectual function. CCAS challenges the traditional view of the cerebellum being responsible solely for regulation of motor functions. It is now thought that the cerebellum is responsible for monitoring both motor and nonmotor functions. The nonmotor deficits described in CCAS are believed to be caused by dysfunction in cerebellar connections to the cerebral cortex and limbic system.

Disorders of diminished motivation (DDM) is an umbrella term referring to a group of psychiatric and neurological disorders involving diminished capacity for motivation, will, and affect.

References

  1. 1 2 3 4 5 6 7 8 9 10 Cairns, H; R. C. Oldfield; J.B. Pennybacker; D. Whitteridge (1941). "Akinetic mutism with an epidermoid cyst of the 3rd ventricle". Brain. 64 (4): 273–290. doi: 10.1093/brain/64.4.273 .
  2. 1 2 3 4 5 6 7 Otto, Anke; Inga Zerr; Maria Lantsch (1998). "Akinetic mutism as a classification criterion for the diagnosis of Creutzfeldt–Jakob Disease". Journal of Neurology, Neurosurgery, and Psychiatry. 64 (4): 524–528. doi:10.1136/jnnp.64.4.524. PMC   2170038 . PMID   9576547.
  3. 1 2 3 4 5 6 7 8 9 10 11 Nagaratnam, Nages; Kujan Nagaratnam; Kevin Ng; Patrick Diu (2004). "Akinetic mutism following stroke". Journal of Clinical Neuroscience. 11 (1): 25–30. doi:10.1016/j.jocn.2003.04.002. PMID   14642361. S2CID   36669323.
  4. Sacks, Oliver (1973). Awakenings. United States: Vintage Books. p. 7.
  5. 1 2 3 Nagaratnam, Nages; Catriona McNeil; J.S. Gilhotra (1999). "Akinetic mutism and mixed transcortical aphasia following left thalamo-mesencephalic infarction". Journal of the Neurological Sciences. 163 (1): 70–73. doi:10.1016/s0022-510x(98)00329-3. PMID   10223414. S2CID   26188424.
  6. 1 2 3 4 5 6 Yarns, Brandon; Davin Quinn (2013). "Telephone Effect in Akinetic Mutism From Traumatic Brain Injury". Psychosomatics. 54 (6): 1–2. doi: 10.1016/j.psym.2013.06.006 . PMID   23932533.
  7. Fix JD. Neuroanatomy. 4th ed.
  8. Struss, DT; Alexander MP; Shallice T; Picton TW; Binns MA; Macdonald R; Borowiec A; Katz DI. (2005). "Multiple frontal systems controlling response speed". Neuropsychologia. 43 (3): 396–417. doi:10.1016/j.neuropsychologia.2004.06.010. PMID   15707616. S2CID   9821228.
  9. Vachalová, Ivana; Viola Gindl; Josef G. Heckmann (August 2013). "Acute inferior homonymous quandrantanopia in a 71-year-old woman". Journal of Clinical Neuroscience. 21 (4): 683–685. doi:10.1016/j.jocn.2013.05.015. PMID   24210803. S2CID   22692004.
  10. Kotchoubey, Boris; Manfred Schneck; Simone Lang; Niels Birbaumer (2003). "Event-related brain potentials in a patient with akinetic mutism". Clinical Neurophysiology. 33 (1): 23–30. doi:10.1016/S0987-7053(03)00003-0. PMID   12711129. S2CID   18789359.
  11. 1 2 Rozen, Todd (2012). "Rapid resolution of akinetic mutism in delayed post-hypoxic leukoencephalopathy with intravenous magnesium sulfate". NeuroRehabilitation. 30 (4): 329–332. doi:10.3233/nre-2012-0763. PMID   22672948.
  12. Yang, Chun-Pai; Huang, Shih; Lin, Lu; Kao, Hsieh; Huang, Lee Tsai (2007). "Diminution of basal ganglia dopaminergic function may play an important role in the generation of akinetic mutism in a patient with anterior cerebral arterial infarct". Clinical Neurology and Neurosurgery. 109 (7): 602–606. doi:10.1016/j.clineuro.2007.04.012. PMID   17543443. S2CID   24185020.